Korean Circ J.  2014 Sep;44(5):348-350. 10.4070/kcj.2014.44.5.348.

Intermittent Pre-Excitation-Syndrome in Facio-Scapulo-Humeral Muscular Dystrophy

• Finsterer
• Stollberger C
• Gatterer E
• Jakubiczka S

• KMID: 2223875
• DOI: http://doi.org/10.4070/kcj.2014.44.5.348

Abstract

Pre-excitation-syndrome has not been reported as a phenotypic feature of facio-scapulo-humeral muscular dystrophy (FSH-MD). In a 39-year-old male with FSH-MD due to a reduced tandem repeat size in the D4Z4-locus on chromosome 4q35, cardiac involvement, manifesting as an incomplete right bundle-branch-block, tall T-waves in V 3-5, ST-elevation in V 2-4, and mild thickening of the left ventricular myocardium, was first recognised 10 years earlier. Follow-up at age 39 years revealed mild myocardial thickening, two intra-ventricular aberrant bands, and, surprisingly, intermittent pre-excitation on a routine electrocardiography. Cardiac involvement in FSH-MD may manifest as hypertrophic cardiomyopathy or various arrhythmias, of which one may be pre-excitation-syndrome.

Keyword

Heart; Muscular dystrophy, Fasuoscapulohumerales; Arrhythmia; Pre-excitation syndromes; Cardiomyopathies

MeSH Terms

Adult
Arrhythmias, Cardiac
Cardiomyopathies
Cardiomyopathy, Hypertrophic
Electrocardiography
Follow-Up Studies
Heart
Humans
Male
Muscular Dystrophies*
Myocardium
Pre-Excitation Syndromes
Tandem Repeat Sequences

Figure

• 

  Fig. 1 Hybridization of a southern blot of the patient's DNA with radioactively labelled probe p13E-11 (D4F104S1); lane 1: DNA cleaved by Eco RI, lane 2: DNA cleaved by Eco RI+Bln I, lane 3: Lambda Mix Marker, 19 (Thermo Scientific Molecular Biology).

• 

  Fig. 2 Routine electrocardiography showing intermittent pre-excitation-syndrome interrupted by QRS-complexes without delta-wave. Three beats are normally conducted (arrows).

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